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Primary Diffuse Large B-Cell Lymphoma
Miral D. Jhaveri, MD, MBA
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            • Diagnostic Checklist

              TERMINOLOGY

              • Abbreviations

                • Primary CNS lymphoma (PCNSL)
                • Diffuse large B-cell lymphoma (DLBCL)
              • Definitions

                • PCNSL: Extranodal lymphoma confined to CNS with absence of systemic disease at time of diagnosis
                • DLBCL: Extranodal DLBCL confined to CNS
                  • Excluded lymphomas arising in dura, intravascular large B-cell, T-cell, NK-cell, secondary CNS lymphomas

              IMAGING

              • General Features

                • CT Findings

                  • MR Findings

                    • Nuclear Medicine Findings

                      • Imaging Recommendations

                        DIFFERENTIAL DIAGNOSIS

                          PATHOLOGY

                          • General Features

                            • Gross Pathologic & Surgical Features

                              • Microscopic Features

                                CLINICAL ISSUES

                                • Presentation

                                  • Demographics

                                    • Natural History & Prognosis

                                      • Treatment

                                        DIAGNOSTIC CHECKLIST

                                        • Consider

                                          • Image Interpretation Pearls

                                            Selected References

                                            1. Bödör C et al: Molecular subtypes and genomic profile of primary central nervous system lymphoma. J Neuropathol Exp Neurol. 79(2):176-83, 2020
                                            2. Green K et al: Cancer, central nervous system lymphoma, 2020
                                            3. Lin X et al: Atypical radiological findings of primary central nervous system lymphoma. Neuroradiology. 62(6): 669-76, 2020
                                            4. Siegal T et al: Primary CNS lymphoma in the elderly: the challenge. Acta Haematol. 141(3):138-45, 2019
                                            5. Lukas RV et al: Diagnostic evaluation in primary CNS lymphoma. Neurologist. 23(2):53-4, 2018
                                            6. Grommes C et al: Primary CNS lymphoma. J Clin Oncol. 35(21):2410-8, 2017
                                            7. Lu SS et al: Histogram analysis of apparent diffusion coefficient maps for differentiating primary CNS lymphomas from tumefactive demyelinating lesions. AJR Am J Roentgenol. 204(4):827-34, 2015
                                            8. Matinella A et al: Neurological complications of HIV infection in pre-HAART and HAART era: a retrospective study. J Neurol. 262(5):1317-27, 2015
                                            9. Yamasaki F et al: Magnetic resonance spectroscopy detection of high lipid levels in intraaxial tumors without central necrosis: a characteristic of malignant lymphoma. J Neurosurg. 122(6):1370-9, 2015
                                            10. Kickingereder P et al: Evaluation of microvascular permeability with dynamic contrast-enhanced MRI for the differentiation of primary CNS lymphoma and glioblastoma: radiologic-pathologic correlation. AJNR Am J Neuroradiol. 35(8):1503-8, 2014
                                            11. Mansour A et al: MR imaging features of intracranial primary CNS lymphoma in immune competent patients. Cancer Imaging. 14:22, 2014
                                            12. Lee IH et al: Analysis of perfusion weighted image of CNS lymphoma. Eur J Radiol. 76(1):48-51, 2010
                                            13. Angelov L et al: Blood-brain barrier disruption and intra-arterial methotrexate-based therapy for newly diagnosed primary CNS lymphoma: a multi-institutional experience. J Clin Oncol. 27(21):3503-9, 2009
                                            14. Correa DD et al: Prospective cognitive follow-up in primary CNS lymphoma patients treated with chemotherapy and reduced-dose radiotherapy. J Neurooncol. 91(3):315-21, 2009
                                            15. Imai H et al: Comparative clinicopathological study of primary CNS diffuse large B-cell lymphoma and intravascular large B-cell lymphoma. Pathol Int. 59(7):431-7, 2009
                                            16. Kim DS et al: Distinguishing tumefactive demyelinating lesions from glioma or central nervous system lymphoma: added value of unenhanced CT compared with conventional contrast-enhanced MR imaging. Radiology. 251(2):467-75, 2009
                                            17. Liao W et al: Differentiation of primary central nervous system lymphoma and high-grade glioma with dynamic susceptibility contrast-enhanced perfusion magnetic resonance imaging. Acta Radiol. 50(2):217-25, 2009
                                            18. Sierra del Rio M et al: Primary CNS lymphoma in immunocompetent patients. Oncologist. 14(5):526-39, 2009
                                            19. Fischer L et al: Meningeal dissemination in primary CNS lymphoma: prospective evaluation of 282 patients. Neurology. 71(14):1102-8, 2008
                                            20. Kleinschmidt-DeMasters BK et al: Epstein Barr virus-associated primary CNS lymphomas in elderly patients on immunosuppressive medications. J Neuropathol Exp Neurol. 67(11):1103-11, 2008
                                            21. Mohile NA et al: The utility of body FDG PET in staging primary central nervous system lymphoma. Neuro Oncol. 10(2):223-8, 2008
                                            22. Toh CH et al: Primary cerebral lymphoma and glioblastoma multiforme: differences in diffusion characteristics evaluated with diffusion tensor imaging. AJNR Am J Neuroradiol. 29(3):471-5, 2008
                                            23. Louis DN et al: WHO Classification of Tumours of the Central Nervous System: Malignant lymphomas. IARC Press. 188-92, 2007
                                            24. Abrey LE et al: Primary central nervous system lymphoma: the Memorial Sloan-Kettering Cancer Center prognostic model. J Clin Oncol. 24(36):5711-5, 2006
                                            25. Hunt MA et al: Distinguishing primary central nervous system lymphoma from other central nervous system diseases: a neurosurgical perspective on diagnostic dilemmas and approaches. Neurosurg Focus. 21(5):E3, 2006
                                            26. Iwamoto FM et al: Primary dural lymphomas: a review. Neurosurg Focus. 21(5):E5, 2006
                                            27. Stadnik TW et al: Diffusion-weighted MR imaging of intracerebral masses: comparison with conventional MR imaging and histologic findings. AJNR Am J Neuroradiol. 22(5):969-76, 2001
                                            28. Bataille B et al: Primary intracerebral malignant lymphoma: report of 248 cases. J Neurosurg. 92(2):261-6, 2000
                                            29. Schlegel U et al: Primary CNS lymphoma: clinical presentation, pathological classification, molecular pathogenesis and treatment. J Neurol Sci. 181(1-2): 1-12, 2000
                                            30. Koeller KK et al: Primary central nervous system lymphoma: radiologic-pathologic correlation. Radiographics. 17(6): 1497-526, 1997
                                            Related Anatomy
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                                            Related Differential Diagnoses
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                                            References
                                            Tables

                                            Tables

                                            KEY FACTS

                                            • Terminology

                                              • Imaging

                                                • Top Differential Diagnoses

                                                  • Pathology

                                                    • Clinical Issues

                                                      • Diagnostic Checklist

                                                        TERMINOLOGY

                                                        • Abbreviations

                                                          • Primary CNS lymphoma (PCNSL)
                                                          • Diffuse large B-cell lymphoma (DLBCL)
                                                        • Definitions

                                                          • PCNSL: Extranodal lymphoma confined to CNS with absence of systemic disease at time of diagnosis
                                                          • DLBCL: Extranodal DLBCL confined to CNS
                                                            • Excluded lymphomas arising in dura, intravascular large B-cell, T-cell, NK-cell, secondary CNS lymphomas

                                                        IMAGING

                                                        • General Features

                                                          • CT Findings

                                                            • MR Findings

                                                              • Nuclear Medicine Findings

                                                                • Imaging Recommendations

                                                                  DIFFERENTIAL DIAGNOSIS

                                                                    PATHOLOGY

                                                                    • General Features

                                                                      • Gross Pathologic & Surgical Features

                                                                        • Microscopic Features

                                                                          CLINICAL ISSUES

                                                                          • Presentation

                                                                            • Demographics

                                                                              • Natural History & Prognosis

                                                                                • Treatment

                                                                                  DIAGNOSTIC CHECKLIST

                                                                                  • Consider

                                                                                    • Image Interpretation Pearls

                                                                                      Selected References

                                                                                      1. Bödör C et al: Molecular subtypes and genomic profile of primary central nervous system lymphoma. J Neuropathol Exp Neurol. 79(2):176-83, 2020
                                                                                      2. Green K et al: Cancer, central nervous system lymphoma, 2020
                                                                                      3. Lin X et al: Atypical radiological findings of primary central nervous system lymphoma. Neuroradiology. 62(6): 669-76, 2020
                                                                                      4. Siegal T et al: Primary CNS lymphoma in the elderly: the challenge. Acta Haematol. 141(3):138-45, 2019
                                                                                      5. Lukas RV et al: Diagnostic evaluation in primary CNS lymphoma. Neurologist. 23(2):53-4, 2018
                                                                                      6. Grommes C et al: Primary CNS lymphoma. J Clin Oncol. 35(21):2410-8, 2017
                                                                                      7. Lu SS et al: Histogram analysis of apparent diffusion coefficient maps for differentiating primary CNS lymphomas from tumefactive demyelinating lesions. AJR Am J Roentgenol. 204(4):827-34, 2015
                                                                                      8. Matinella A et al: Neurological complications of HIV infection in pre-HAART and HAART era: a retrospective study. J Neurol. 262(5):1317-27, 2015
                                                                                      9. Yamasaki F et al: Magnetic resonance spectroscopy detection of high lipid levels in intraaxial tumors without central necrosis: a characteristic of malignant lymphoma. J Neurosurg. 122(6):1370-9, 2015
                                                                                      10. Kickingereder P et al: Evaluation of microvascular permeability with dynamic contrast-enhanced MRI for the differentiation of primary CNS lymphoma and glioblastoma: radiologic-pathologic correlation. AJNR Am J Neuroradiol. 35(8):1503-8, 2014
                                                                                      11. Mansour A et al: MR imaging features of intracranial primary CNS lymphoma in immune competent patients. Cancer Imaging. 14:22, 2014
                                                                                      12. Lee IH et al: Analysis of perfusion weighted image of CNS lymphoma. Eur J Radiol. 76(1):48-51, 2010
                                                                                      13. Angelov L et al: Blood-brain barrier disruption and intra-arterial methotrexate-based therapy for newly diagnosed primary CNS lymphoma: a multi-institutional experience. J Clin Oncol. 27(21):3503-9, 2009
                                                                                      14. Correa DD et al: Prospective cognitive follow-up in primary CNS lymphoma patients treated with chemotherapy and reduced-dose radiotherapy. J Neurooncol. 91(3):315-21, 2009
                                                                                      15. Imai H et al: Comparative clinicopathological study of primary CNS diffuse large B-cell lymphoma and intravascular large B-cell lymphoma. Pathol Int. 59(7):431-7, 2009
                                                                                      16. Kim DS et al: Distinguishing tumefactive demyelinating lesions from glioma or central nervous system lymphoma: added value of unenhanced CT compared with conventional contrast-enhanced MR imaging. Radiology. 251(2):467-75, 2009
                                                                                      17. Liao W et al: Differentiation of primary central nervous system lymphoma and high-grade glioma with dynamic susceptibility contrast-enhanced perfusion magnetic resonance imaging. Acta Radiol. 50(2):217-25, 2009
                                                                                      18. Sierra del Rio M et al: Primary CNS lymphoma in immunocompetent patients. Oncologist. 14(5):526-39, 2009
                                                                                      19. Fischer L et al: Meningeal dissemination in primary CNS lymphoma: prospective evaluation of 282 patients. Neurology. 71(14):1102-8, 2008
                                                                                      20. Kleinschmidt-DeMasters BK et al: Epstein Barr virus-associated primary CNS lymphomas in elderly patients on immunosuppressive medications. J Neuropathol Exp Neurol. 67(11):1103-11, 2008
                                                                                      21. Mohile NA et al: The utility of body FDG PET in staging primary central nervous system lymphoma. Neuro Oncol. 10(2):223-8, 2008
                                                                                      22. Toh CH et al: Primary cerebral lymphoma and glioblastoma multiforme: differences in diffusion characteristics evaluated with diffusion tensor imaging. AJNR Am J Neuroradiol. 29(3):471-5, 2008
                                                                                      23. Louis DN et al: WHO Classification of Tumours of the Central Nervous System: Malignant lymphomas. IARC Press. 188-92, 2007
                                                                                      24. Abrey LE et al: Primary central nervous system lymphoma: the Memorial Sloan-Kettering Cancer Center prognostic model. J Clin Oncol. 24(36):5711-5, 2006
                                                                                      25. Hunt MA et al: Distinguishing primary central nervous system lymphoma from other central nervous system diseases: a neurosurgical perspective on diagnostic dilemmas and approaches. Neurosurg Focus. 21(5):E3, 2006
                                                                                      26. Iwamoto FM et al: Primary dural lymphomas: a review. Neurosurg Focus. 21(5):E5, 2006
                                                                                      27. Stadnik TW et al: Diffusion-weighted MR imaging of intracerebral masses: comparison with conventional MR imaging and histologic findings. AJNR Am J Neuroradiol. 22(5):969-76, 2001
                                                                                      28. Bataille B et al: Primary intracerebral malignant lymphoma: report of 248 cases. J Neurosurg. 92(2):261-6, 2000
                                                                                      29. Schlegel U et al: Primary CNS lymphoma: clinical presentation, pathological classification, molecular pathogenesis and treatment. J Neurol Sci. 181(1-2): 1-12, 2000
                                                                                      30. Koeller KK et al: Primary central nervous system lymphoma: radiologic-pathologic correlation. Radiographics. 17(6): 1497-526, 1997